In addition to those initially described, other monogenic syndromes were recognized, including proteasome-associated autoinflammatory syndromes (PRAAS), ISG15 deficiency, Singleton–Merten syndrome (SMS), and STING-associated vasculopathy with onset in infancy (SAVI) (43, 44). The gene discussed is STING1; the disease is proteosome-associated autoinflammatory syndrome.